| RRC ID |
1857
|
| 著者 |
Inoue S, Ishikawa K, Nakada K, Sato A, Miyoshi H, Hayashi J.
|
| タイトル |
Suppression of disease phenotypes of adult mito-mice carrying pathogenic mtDNA by bone marrow transplantation.
|
| ジャーナル |
Hum Mol Genet
|
| Abstract |
For directly addressing the issue of gene therapy of adult patients with mitochondrial diseases, we carried out bone marrow transplantation to adult mito-mice with mutated mtDNA and expressing respiration defects for improvement of disease phenotypes. We supposed that bone marrow cells transdifferentiated into various tissues, so that their transplantation would suppress disease phenotypes. The results showed improvement of survival and delayed expression of renal failure. As most mito-mice without a transplant died due to renal failure, we examined whether transplanted bone marrow cells transdifferentiated into renal tissues carrying improved renal function. Histochemical analyses showed that the suppression of disease phenotypes was not due to transdifferentiation, but due to suppression of apoptosis of renal cells. Thus, bone marrow cells possess a novel function of supporting tissues by suppressing apoptosis induced by respiration defects.
|
| 巻・号 |
15(11)
|
| ページ |
1801-7
|
| 公開日 |
2006-6-1
|
| DOI |
10.1093/hmg/ddl102
|
| PII |
ddl102
|
| PMID |
16613898
|
| MeSH |
Animals
Apoptosis
Bone Marrow Transplantation / methods*
Cell Differentiation
DNA, Mitochondrial* / metabolism
Genetic Therapy / methods
Immunohistochemistry
In Situ Nick-End Labeling
Male
Mice
Mice, Transgenic
Mutation
Phenotype
Renal Insufficiency / metabolism
|
| IF |
5.101
|
| 引用数 |
7
|
|
WOS 分野
|
GENETICS & HEREDITY
BIOCHEMISTRY & MOLECULAR BIOLOGY
|
| リソース情報 |
| 実験動物マウス |
RBRC00267
RBRC01603 |
